Iwona Stelmach, Aleksandra Korzeniewska, Joanna Jerzyńska
CaseRepClinPractRev 2007; 8:83-85
Background: There are few postmortem studies of the heart in CF patients, but lipomatosis cordis has not been described in this group.
Case Report: We report a case of fatty infiltration of the heart, found in postmortem study in adult patient with cystic fibrosis (CF) who died of multiorgan trauma after car crash. During his whole life, he had malnutrition, with BMI significantly lower than for his age. Our patient was very well adopted to chronic hypoxia, he did not demonstrate symptoms of right
ventricular disease, but his pulmonary function was severly impaired. None clinical signs of heart disease were observed. Autopsy showed the 270 g weight heart, sized 90x90x50 mm with excessively developed subepicardial yellow fatty tissue penetrating into the myofibers. Microscopic examination showed lipomatosis cordis and fatty degeneration and no signs of necrosis.
Conclusions: Lipomatosis cordis with fatty degeneration has not been described in patients with cystic fibrosis and finding of this abnormality in CF patient was unexpected. We can not exclude the influence of glicocorticosteroids treatment together with high lipid diet on development
of described fatty infiltration of the heart. Following this hypothesis the lipomatosis cordis in our case could be a side effect of CF. Taking these findings into account, CF patients should be regularly examined with laboratory tests even if there are no clinical symptoms of heart disease. However, we can not rule out, that unknown changes in lipid metabolism
in this patient, resulted in development of lipomatosis cordis nor our case report describes a coincidental finding.
Keywords: lipomatosis cordis, Cystic Fibrosis